Transcript Autoimmune Inner Ear Disease

Autoimmune Inner Ear Disease
Robert H. Stroud, M.D.
Jeffery T. Vrabec, M.D.
12 January 2000
Background
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Lenhardt 1958
McCabe 1979
Harris 1990
Immune Function of Inner Ear
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blood-labyrinthine barrier
maintenance of homeostasis
little lymphatic drainage
immunoglobulins 1/1000th of serum
immune responsiveness
Endolymphatic Sac
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Resident lymphocytes
immunoglobulin production
systemic lymphocyte entry
– spiral modiolar vein
– intercellular adhesion molecule
Type I Hypersensitivity
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IgE
mast cells
histamine
vasodilation
? Hydrops
Meniere’s
inhalant allergy
Type II Hypersensitivity
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Antibodies
complement
activation
anti-68kDa protein
antibody
SLE, Goodpasture’s
Type III Hypersensitivity
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Immune complex
Ig deposition
tissue injury
Wegener’s,
?Meniere’s
Type IV Hypersensitivity
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T-cell mediated
direct lysis
lymphokine
production
lymphocyte
transformation test
Cogan’s syndrome
Clinical Picture
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Middle-aged women
progressive SNHL, weeks to months
dizziness, aural fullness
bilateral 79%
 no vestibular symptoms
systemic autoimmune disease in 29%
Diagnosis
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Clinical
LTT - 93% specific, 50-80% sensitive
Western blot for anti-68kDa protein
(hsp70)
– 95% specific
– insensitive
– predictor of steroid response
Diagnosis
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ESR
CRP
C1q binding assay
anti-cardiolipin
ANCA
syphilis testing
Lyme titers
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CBC
chemistries
thyroid functions
imaging
Polyarteritis Nodosa
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Vasculitis of small and medium-sized
arteries
renal and visceral
ischemia  osteoneogenesis  fibrosis
hearing loss rare
Cogan’s Syndrome
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Interstitial keratitis
vertigo, tinnitus,
SNHL
positive LTT to
corneal antigen
Vogt-Koyanagi-Harada (VKH)
Syndrome
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SNHL, vestibular signs, uveitis
periorbital hair loss, depigmentation
aseptic meningitis
?autoimmunity to melanocytes
Wegener’s Granulomatosis
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Necrotizing
granulomata
vasculitis
respiratory tract and
kidneys
serous OM
cANCA 90% specific
Behçet’s Disease
Relapsing Polychondritis
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Recurrent
inflammation of ear,
nose, trachea,
larynx
autoantibodies to
cartilage II & IX
NSAIDs, steroids,
dapsone
Systemic Lupus Erythematosus
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Anti-nuclear, antiDNA antibodies
numerous systemic
manifestations
COM with vasculitis,
SNHL,
dysequilibrium
Rheumatoid Arthritis
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Small joints of hands and feet
vasculitis, muscle atrophy,
subcutaneous nodules, splenomegaly
IgM 19S and 7S, IgG 7S 75%
44% bilateral SNHL
Meniere’s Disease
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Fluctuating SNHL,
episodic vertigo,
aural fullness
? Autoimmune
etiology
– 97% with CICs
(Derebery)
– response to
immunotherapy
– 32% with anti-68kDa
antibody
Treatment
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Steroids
Cyclophosphamide
Plasmapheresis
Methotrexate
– dihydrofolate reductase inhibitor
Complications of therapy
Case Study
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45 year old female
right sided hearing loss and aural
fullness, dysequilibrium progressive
over 2 months time
physical normal except Weber AS,
Rinne positive AU
Case Study, continued
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CBC, chemistries,
TFTs, RPR, ESR
normal
MRI acoustic
protocol normal
low salt diet,
Dyazide
Case Study, continued
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At follow-up, AD
hearing worse
Prednisone 30 mg
BID
anti-68kDa protein
positive
Case Study, continued
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Hearing improved
steroids tapered
one relapse, again
with improvement
on steroids
Conclusion
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Elusive etiology, diagnosis and
treatment
potentially treatable cause of
progressive SNHL
need less toxic therapy